{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T02:46:45Z","timestamp":1790909205454,"version":"4.1.0"},"reference-count":83,"publisher":"SAGE Publications","issue":"3","license":[{"start":{"date-parts":[[2010,9,27]],"date-time":"2010-09-27T00:00:00Z","timestamp":1285545600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["World J Pediatr Congenit Heart Surg"],"published-print":{"date-parts":[[2010,10]]},"abstract":"<jats:p> Fontan\u2019s visionary operation and its modifications over the ensuing decades have re-established nonturbulent flow and substantially reduced cyanosis for patients with severe hypoplasia of one ventricle. However, a long list of largely unexpected sequelae has emerged over the last 40 years. Although it is not difficult to understand how care providers could become discouraged, a number of myths have arisen, which we will attempt to dispel with real-world counterexamples as well as with lessons learned from other disciplines: evolutionary, developmental, and computational biology. We argue that distinctive biochemical abnormalities pointing to dysfunction in multiple organs, including the largest organ system in the body, the endothelium, occur long before grossly observable changes in cardiac imaging can be recognized. With a rational redesign of both our surveillance scheme and our wellness strategies, we hope that Fontan survivors and their families, as well as physicians, nurses, and therapists, will see why Fontan\u2019s principle remains just as vibrant today as it was in 1971. <\/jats:p>","DOI":"10.1177\/2150135110379623","type":"journal-article","created":{"date-parts":[[2010,9,27]],"date-time":"2010-09-27T22:39:39Z","timestamp":1285627179000},"page":"328-343","source":"Crossref","is-referenced-by-count":17,"title":["Insights After 40 Years of the Fontan Operation"],"prefix":"10.1177","volume":"1","author":[{"given":"Alvin J.","family":"Chin","sequence":"first","affiliation":[{"name":"Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania"},{"name":"Division of Cardiology, Children\u2019s Hospital of Philadelphia, Philadelphia, Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kevin K.","family":"Whitehead","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania"},{"name":"Division of Cardiology, Children\u2019s Hospital of Philadelphia, Philadelphia, Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raymond L.","family":"Watrous","sequence":"additional","affiliation":[{"name":"Department of Electrical and Systems Engineering, University of Pennsylvania School of Engineering and Applied Science, Philadelphia, Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2010,9,27]]},"reference":[{"key":"bibr1-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1136\/thx.26.3.240"},{"key":"bibr2-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1053\/j.pcsu.2010.01.006"},{"key":"bibr3-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1053\/j.pcsu.2010.01.002"},{"key":"bibr4-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.107.738559"},{"key":"bibr5-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1007\/s11136-008-9323-8"},{"key":"bibr6-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-5223(96)70051-X"},{"key":"bibr7-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjcard.2007.08.029"},{"key":"bibr8-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1053\/j.pcsu.2004.02.008"},{"key":"bibr9-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-5223(98)70406-4"},{"key":"bibr10-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/0735-1097(93)90836-P"},{"key":"bibr11-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.90.6.2927"},{"key":"bibr12-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCIMAGING.108.834192"},{"key":"bibr13-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCIMAGING.108.832113"},{"key":"bibr14-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1017\/S1047951100008118"},{"key":"bibr15-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1007\/s00431-005-0030-y"},{"key":"bibr16-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1007\/BF02238376"},{"key":"bibr17-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2008.03.077"},{"key":"bibr18-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.65.6.1043"},{"issue":"11","key":"bibr19-2150135110379623","first-page":"I165","volume":"116","author":"Whitehead KK","year":"2007","journal-title":"Circulation"},{"key":"bibr20-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2008.11.062"},{"key":"bibr21-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1053\/j.pcsu.2010.02.006"},{"key":"bibr22-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2004.04.013"},{"key":"bibr23-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/1010-7940(89)90088-2"},{"key":"bibr24-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/j.echo.2009.06.003"},{"key":"bibr25-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejcts.2007.11.012"},{"key":"bibr26-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9149(01)02159-2"},{"key":"bibr27-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1017\/S1047951109990382"},{"key":"bibr28-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1097\/SLA.0b013e3181858286"},{"key":"bibr29-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejcts.2007.12.032"},{"key":"bibr30-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejcts.2006.11.043"},{"key":"bibr31-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2009.10.050"},{"key":"bibr32-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-009-9612-1"},{"key":"bibr33-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1101\/gad.13.8.1037"},{"key":"bibr34-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1038\/344879a0"},{"key":"bibr35-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/S1534-5807(03)00363-0"},{"key":"bibr36-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1038\/nature08191"},{"key":"bibr37-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2007.01.042"},{"key":"bibr38-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/j.ydbio.2007.08.004"},{"key":"bibr39-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.00093-07"},{"key":"bibr40-2150135110379623","unstructured":"University of Washington. Biology 453: Comparative Vertebrate Anatomy. Open access lab notes. Available at: http:\/\/courses.washington.edu\/chordate\/453labs\/453lab13-2010.pdf. Accessed June 1, 2010."},{"key":"bibr41-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1007\/978-94-009-8624-4_4"},{"key":"bibr42-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1006\/dbio.1999.9298"},{"key":"bibr43-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1038\/nature08324"},{"key":"bibr44-2150135110379623","unstructured":"Kolditz DP. Cardiac development in relation to clinical supraventricular arrhythmias : focus on structure-function relations [Theses]. Open access publication in the Leiden University Repository. Available at: https:\/\/openaccess.leidenuniv.nl\/bitstream\/1887\/13721\/7\/01.pdf. Accessed June 1, 2010."},{"key":"bibr45-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(01)00493-7"},{"key":"bibr46-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1089\/zeb.2004.1.111"},{"key":"bibr47-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2656.2006.01201.x"},{"key":"bibr48-2150135110379623","doi-asserted-by":"publisher","DOI":"10.2307\/1447765"},{"key":"bibr49-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-010X(19960601\/15)275:2\/3<112::AID-JEZ5>3.0.CO;2-G"},{"key":"bibr50-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1002\/ar.10126"},{"key":"bibr51-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/S0003-4975(03)01173-1"},{"key":"bibr52-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/S0003-4975(01)02693-5"},{"key":"bibr53-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/0735-1097(92)90157-I"},{"key":"bibr54-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcmg.2009.03.019"},{"key":"bibr55-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1007\/s00247-009-1508-y"},{"key":"bibr56-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2008.02.081"},{"key":"bibr57-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1007\/s10558-007-9025-9"},{"key":"bibr58-2150135110379623","first-page":"3105","volume":"2009","author":"Watrous RL","year":"2009","journal-title":"Conf Proc IEEE Eng Med Biol Soc"},{"key":"bibr59-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/j.ppedcard.2006.07.007"},{"key":"bibr60-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.72.6.591"},{"key":"bibr61-2150135110379623","doi-asserted-by":"publisher","DOI":"10.4065\/71.4.378"},{"key":"bibr62-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.96.1.33"},{"key":"bibr63-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1067\/mtc.2001.110681"},{"key":"bibr64-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.2003.029041"},{"key":"bibr65-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-005-0913-8"},{"key":"bibr66-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-008-9292-2"},{"key":"bibr67-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/S0065-2423(10)50003-9"},{"key":"bibr68-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1017\/S1047951110000648"},{"key":"bibr69-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/j.amjcard.2004.08.085"},{"key":"bibr70-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2010.04.037"},{"key":"bibr71-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2009.03.091"},{"key":"bibr72-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1007\/s00247-009-1439-7"},{"key":"bibr73-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2009.03.025"},{"key":"bibr74-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCRESAHA.107.165530"},{"key":"bibr75-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2007-08-107359"},{"key":"bibr76-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9343(88)80066-4"},{"key":"bibr77-2150135110379623","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.175.6.4009"},{"key":"bibr78-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2008.12.065"},{"key":"bibr79-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1111\/j.1747-0803.2009.00320.x"},{"key":"bibr80-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1152\/physrev.00024.2009"},{"key":"bibr81-2150135110379623","doi-asserted-by":"publisher","DOI":"10.2353\/ajpath.2008.080060"},{"key":"bibr82-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1016\/j.jtcvs.2008.09.031"},{"key":"bibr83-2150135110379623","doi-asserted-by":"publisher","DOI":"10.1017\/S1047951107001680"}],"container-title":["World Journal for Pediatric and Congenital Heart Surgery"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/2150135110379623","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/full-xml\/10.1177\/2150135110379623","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/2150135110379623","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,3,3]],"date-time":"2025-03-03T13:49:52Z","timestamp":1741009792000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.1177\/2150135110379623"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010,9,27]]},"references-count":83,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2010,10]]}},"alternative-id":["10.1177\/2150135110379623"],"URL":"https:\/\/doi.org\/10.1177\/2150135110379623","relation":{},"ISSN":["2150-1351","2150-136X"],"issn-type":[{"value":"2150-1351","type":"print"},{"value":"2150-136X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2010,9,27]]}}}